Discontiguous or Contiguous Spread Patterns Affect the Functional Staging in Patients With Sporadic Amyotrophic Lateral Sclerosis.

Li Zhenfei,Zhou Xiaomeng,Duan Shiru,Liu Yaling,Cao Cuifang

doi:10.3389/fneur.2019.00523

Li Zhenfei, Zhou Xiaomeng + Show 3 more

Open Access

https://doi.org/10.3389/fneur.2019.00523

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Abstract

Objective: This study aimed to investigate whether the spread pattern affects functional staging in amyotrophic lateral sclerosis (ALS). We examined the spreading patterns of disease following symptom onset and the affected regions in ALS using electromyography.Methods: This study reviewed the medical records of 103 patients with sporadic ALS in the Second Hospital of Hebei Medical University from 2012 to 2017. According to the clinical manifestation and the distribution of the affected regions on electromyography, spread patterns were classified as discontiguous or contiguous. The patients were graded according to the ALS-Milano-Torino staging (MITOS) system.Results: The clinical spread patterns were contiguous in 91.5% of patients and discontiguous in 8.5% of patients. The electrophysiological spread patterns were contiguous in 87.4% of patients and discontiguous in 12.6% of patients. Sex, age, or delay in diagnosis did not affect the clinical or electrophysiological spread patterns. No significant correlation was observed between the clinical classification and the ALS-MITOS grade, but the electrophysiological spread was significantly correlated with the ALS-MITOS.Conclusion: This study provides evidence that not all ALS patients show contiguous clinical or electrophysiological spread patterns. The electrophysiological spread pattern can affect the functional staging in ALS patients.

Highlights

Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease affecting the motor nervous system
Volunteers participated in the study after giving informed consent, which was approved by our hospital
Patients with a family history of primary lateral sclerosis; clinically suspected ALS or frontotemporal dementia; or severe heart, lung, liver, or kidney diseases were excluded from this study

Summary

Introduction

Amyotrophic lateral sclerosis (ALS) is a fatal degenerative disease affecting the motor nervous system. There are several hypotheses about the pathogenesis of ALS. Prion-like protein aggregation has recently been proposed as a new mechanism of ALS. Prion Protein (PrP) is a type of selfproliferating and contagious cell membrane protein and is widely expressed in the body, especially in the nerve tissues [1]. The prion-like mechanism is considered to involve a misfolded protein that is similar to PrP and can reproduce and accumulate. The prion-like mechanism is one of the pathophysiological mechanisms of neurodegenerative disease. This mechanism appears in ALS and in other neurodegenerative diseases, such as Parkinson’s disease [2] and Alzheimer’s

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